NextGen 2024: Refractory SJIA & MAS Session Part 6
NextGen 2024: Refractory SJIA & MAS Session Part 6
International perspectives (China): Presentation from Dr. Caifeng Li
NextGen 2024: Refractory SJIA & MAS Session Part 6
@SJIA_Foundation2 months ago

Systemic Juvenile Idiopathic Arthritis in China
Caifeng Li, MD, PhD
Professor of Capital Medical University Chief of Pediatric Rheumatology Department, Beijing Children's Hospital, National Medical Center for Children Chief of Rheumatology Department, Capital Medical University Executive President of Beijing Children's Hospital Zhengzhou Hospital
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Treatment of sJIA - glyph
Treatment of sJIA in Beijing Children's Hospital - â sJIA with MAS
- â sJIA with lung involvement
- â refractory sJIA
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Unmet needs and future perspectives - â Glucocorticoids
- â NSAIDs
- â Cyclosporine
- â Thalidomide
- â Tocilizumab
- â JAK inhibitors: Tofacitinib
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Treatment Plan - â general treatment:
- â GC+ NSAIDs+ Cyclosporine+ Thalidomide
- â GC+ NSAIDs+ Tocilizumab
- â refractory sJIA treatment:
- â GC+ NSAIDs+ Cyclosporine+ Thalidomide+ Tocilizumab/ Tofacitinib
- â TNF inhibitors added for patients with chronic arthritis
- â Glucocorticoid: 545 patients (99.8%) initially received, with 501 (91.8%) receiving one dose of MP pulse therapy, 20 received 2 doses MP pulse, 3 received 3 doses MP pulse
- â Most commonly used DMARDs: CSA (96.3%), MTX (8.4%, primarily for arthritis)
- â 41 cases (7.5%) used Thalidomide
- â Biologics and JAK inhibitors: Tocilizumab was the primary choice (16.8%), and 31 cases (5.7%) received Tofacitinib treatment
- â Treatment plan: Glucocorticoids + NSAIDs + DMARDs + biologics/JAK inhibitors
- â At 1 month, 74.2% of patients achieved ACRp30 response
- â At 3 months, 94.2% achieved ACRp30 response
- â By 24 months
- â 100% of patients reached ACRp30 response
- â 95.4% achieved ACRp50 response
- â 75% reached ACRp70 response
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Clinical data of 141 sJIA-MAS cases in BCH - glyph
94 males and 47 females, male-to-female ratio 2:1 - glyph
- Average age of onset: 7.6±3.4 years
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16 (11.3%) cases of onset with MAS - glyph
Clinical Manifestations: the main clinical features included fever (100%), hematologic involvement (95%), and respiratory involvement (90%) - â Respiratory involvement:
- â pleural effusion: 38 cases (27%)
- â acute respiratory distress syndrome: 11 cases (5.7%)
- â 70 (100%) received MP pulse therapy
- â 48 (68.6%) received cyclosporine
- â The most common treatment plan:
- â MP pulse+CSA+ IVIG (38.6%)
- â followed by MP pulse + IVIG (28.6%)
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Data of 280 sJIA cases in BCH, including 38 (13.6%) cases with lung involvement - glyph
male-to-female ratio of 1.5:1, average age of onset: 5.7 ± 3.0 years - glyph
Among patients with lung involvement, 13 (34.2%) cases had MAS - glyph
Types of lung involvement: - â the main type was interstitial lung disease (92.1%)
- â pulmonary hemorrhage (10.5%), pulmonary hypertension (5.3%)
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Clinical Manifestations: fever and respiratory symptoms - â mainly cough and chest tightness, 13 (34.2%) required respiratory support
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- â 37 (97.4%) used GC, with 73.3% receiving MP pulse therapy
- â 17 (44.7%) used cyclosporine
- â 6 (15.8%) added Tocilizumab/JAK inhibitors
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Follow-up: 32 patients for 5-105 months - â 26 (81.3%) showed improvement
- â 22 (68.7%) added tocilizumab
- â 5 (15.6%) added tofacitinib
- â 1 patient stopped all medications
- â 10 cases (31.3%) stopped GC
- â 3 (9.4%) died due to MAS, pulmonary hemorrhage, respiratory failure, ARDS, and renal failure
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Data of 11 refractory sJIA cases in BCH: 7 males, 4 females - â Reasons for relapse:
- â At onset: average age 3.5 years, 5 onset with MAS, 5 had lung involvement
- â MAS (7, 63.6%), 5 patients experienced 2 times of MAS
- â Infection caused disease activity (6, 54.5%)
- â Chronic arthritis (2, 18.2%)
- â Outcomes: 2 give up due to multiple organ failure, 3 achieved disease control with tocilizumab, 5 achieve control with Tofacitinib, and 4 (36.4%) stop GC
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Subsequent Plan - sJIA Database Construction in China - â Construct a nationwide multicenter sJIA database
- â Incorporating 34 tertiary medical institutions
- â Plan to complete the database construction by December 2024

Contents

Treatment of sJIA
glyph Commonly used medications for sJIA in China:

Treatment of sJIA in BCH
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Treatment of sJIA in BCH
glyph Follow-up data of 260 sJIA cases in BCH


sJIA with MAS in BCH


sJIA with MAS in BCH
glyph Treatment data of 70 sJIA-MAS cases in BCH


sJIA with lung involvement in BCH


sJIA with lung involvement in BCH
Initial treatment: GC + NSAIDs + DMARDs


sJIA with lung involvement in BCH
glyph HRCT scans of lungs in some patients during follow-up


Before treatment: sJIA-ILD After treatment (22 months) (Organising pneumonia, OP)
Before treatment: sJIA-ILD+MAS After treatment (5 months)

refractory sJIA in BCH


Unmet needs and future perspectives




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